Exchanges.
Travelling and Socialising.
Morning Sickness.
The first Scan.

Click here to view the embedded video.

Her exchanges went from 2 to 44 during the pregnancy. She explains that babies receive food and nutrients from their mothers. The baby is connected to the placenta by an umbilical cord. This carries the food and nutrients to the baby. Protein is the key building block needed to support a baby’s growth. This is why Angela’s exchanges went up so rapidly.

Being pregnant with PKU does not have to restrict your life. Despite the need to follow such a strict diet, Angela gives an account of how she travelled to Turkey for her sisters wedding.

Morning sickness was a bit of a pain as it sometimes meant having to take a second batch of protein supplements!

The first scan came as a bit of a shock because she discovered she had twins!!!

To see part 1 of Angela’s blog please click here.

Click here to view the embedded video.

An important aspect of the pre-conception diet was the time she spent with her dietitian: revisiting the importance of weighing food properly and being organised. Angela also talks about how her mental alertness improved once she started the diet.

Some useful ideas (relevant to anyone with PKU):

• Keep a food diary.
• Re-think your food shopping – the need for “free” or low protein foods to fill you up.
• Sign up for a home delivery service.
• Fizzy drinks are you friend (especially Lucozade).
• Look for low protein recipes that you can easily make.
• Buy a digital cooking scales – it’s easier and more accurate.

For clarification the term “ pre-conception diet” refers to the need to go on diet prior to conception.

Every couple of months I go to my gp’s clinic where I have a blood test which is sent to a laboratory where it is tested to see the levels of phenylalanine in my blood. This makes it harder to cheat and have things I’m not allowed as it will show up here. Twice a year I see my doctor and my dietician together. We discuss things like my general health and whether I have any problems. My height, weight, and blood pressure are also measured and we discuss my progress. I really enjoy these meetings as they make me feel like I’m in charge of my own diet instead of my mum doing everything for me.

PKU doesn’t stop me doing things I like, I have been on several holidays, both with my family, and with my school. I take my own food and have no difficulty finding something I can eat and enjoy. Most places where I have stayed have been very helpful when I explain my problem.

I used to get picked on by other children for my condition and had to sit by the teachers at lunch so they could keep an eye on what I was having, this made the other kids think I was a teachers pet who wanted to sit with the teachers all the time. AS IF!!! Also when I was younger, there was a time when I wasn’t invited to a friends birthday party. This was because her mum didn’t fully understand my condition and didn’t want to invite me and end up giving me the wrong thing. Eventually my mother went into school and gave a talk on my condition and its effects where the other parents were invited to come along and listen. Since then I’ve actually made more friends because they know I’m not a teachers pet and just want to be treated like everyone else. When a new girl started at our school who is diabetic, I was the first to make friends with her.

One thing I must always look out for is changes in products, especially drinks. This is because manufacturers are always changing the ingredients, adding some or taking them away. This means they can end up on what I call the “red list”.

I do meet other children with PKU. There are special events organized by the NSPKU where can all get together and just have a good time. I find it exciting meeting others with my problems. It makes me feel like there is so much out there I can do, especially when I meet adults with PKU who have families and jobs and don’t let this one thing hold them back, especially as I will have this condition for ever.

PKU has not stopped me having a normal life. Although at times it is a bit inconvenient, I can still enjoy normal activities and my food, within the limitations of my diet.

It was, like last year, a tremendous success with the right mix of fun and education. Some of the highlights included the quiz on Friday night, the Cookery School on Saturday afternoon, Dr. Monivari’s talk on future developments in the treatment of Metabolic disorders, David Coleman’s talk on Sunday (he of RTE fame) advising parents on how to work with their children on lifestyle adjustment, and the Grand National sweepstake which ran on Saturday, including a number of presentations from dietitians and patients on management of PKU and all inborn Metabolic Disorders.

We will be announcing the date for next year’s event in the coming weeks and we hope next years event will be even bigger and better, roll on Low Protein Living Weekend 2010.

The survey is concerned with newborn screening that is undertaken in the first 5-8 days of a baby’s life (the ‘heel prick’ test).  From the blood sample taken it is possible to recognise some disorders early in a baby’s life which if left untreated would cause serious health problems. As part of a research project it is proposed to extend screening to test for additional inherited metabolic diseases.

The survey is open to anyone but we are especially interested in the unique views of parents of children affected by inherited metabolic conditions.

We are interested in your views on how information is given about this testing and how it could be improved. This is not a test and there are no right or wrong answers!

Once you have completed the survey you will be given the opportunity to enter into a free prize draw for £100 worth of vouchers! It should take about 20 minutes of your time.

The link to the online survey is:

http://www.surveymonkey.com/s.aspx?sm=_2byOu2FjRu9kbR_2fgN8AssKA_3d_3d

If you have any queries about this research please email Louise Moody (L.Moody@coventry.ac.uk).

As a kid, I was eating mainly carbs and fats and lots of fruit, but very small amounts. I was a tearaway with ADHD so I was burning through energy like no tomorrow. As a result, I was a pretty lean kid and my dieticians expressed concern that while I was a tall child, I was pretty light.

As a result, I was put given Calogen supplements which I took in the form of a milkshake with a beautiful zero-protein fruit ice cream called Nectacream. While the Calogen suppliments had their desired effect (I went from a lean skinny lanky kid to a reasonably solid and well-padded youngster) the company that made Nectacream folded, and that was the end of that. It saddens me that Nectacream doesn’t exist today – I’m sure it would have made a killing as a PKU ice-cream if it had gotten the global exp

osure the internet may have been able to afford it. Rest in cold, creamy peace, my long-lost friend.

Anyway, I was around 73 kilos going into college and struggling with my diet. I was taking my Aminogran supplement less and less because the morning doses made my breath smell horrible and I quickly spun out of the habit. In a bid to teach me some responsibility, my mother made me make it for myself. It backfired and I just stopped.

So I went off diet for a while – approximately five years. During that time I found college extremely difficult, and my life was somewhat turbulent. Needless to say, I made it through college and into university. My first year was typical – wild parties, lots of alcohol and lots of bar food – I developed a ‘healthy’ taste for garlic bread and fries, and spent many a sunny afternoon with my textbooks, beer and snack food. After a fun season during my final year of college, I went back to rugby and played in an Under 85 kilo weight grade. Due to my lifestyle and my regular dinner (pork crackling + mash potato + oven fries) I put on a lot of weight, and soon found myself weighing out of my games. After some problems with my then on-again off-again girlfriend and being forced to watch most of my team’s game from the sideline, I became pretty down. The fact I was not getting anywhere near what my body needed in terms of nutrition was no doubt a contributing factor.

Things culminated when a series of high blood test levels and my regular appointment at our national metabolic clinic saw me faced with the very real possibility that I was doing myself serious damage, and my B12 levels were a cause for concern. I knew I had to take my Amingran more seriously, but I could not get myself into the habit of making it. Thankfully, I was introduced to Easiphen, which our national pharmalogical body was contemplating funding.

In the four years that have followed, I have gone from being disorganised, irresponsible, clueless, incapable and dangerously incompetent with my diet to having a really good handle on it. My extra weight, which was putting me at risk of a series of other problems such as high cholesterol and diabetes is now firmly under control. I have regained a svelter figure through an aggressive low-cal diet with rigid Easiphen intake and have re-registered for the Under 85 team I had to effectively walk away from in 2005. I’ve gone from 103 kilos to around 81kgs, and there’s more to come off. I’m also trying to build some muscle mass, although the low protein diet is proving to be a bit of a stumbling block in this regard. If anyone knows of any good suppliments for this purpose, please message me! I love to cook and while I am getting very good at making breads and pizzas, I also enjoy some treats like popcorn in grapeseed oil (absolutely beautiful!).

I’m proud to have PKU. It’s no longer a stumbling bloc, it’s something that I know know a lot about and have read many things on. Understanding my condition has helped me take care of myself and given me something to be proud of.

I’m sorry for how long this is, but there is a silver lining to going back on the diet. If you’re on it now as a teen, for the love of all things awesome, stay on it! Learn to cook for yourself using things like LoProfin and discover the small wonderful things about your condition that other people don’t get to experience. Most other people will rarely smell bread fresh out of an oven – PKU lets you do that all the time! You can also make scones, pizzas and all sorts of wonderful goodies with relative impunity! I love the taste of my baking, I look forward to mucking around with recipes and it’s pretty much the most rewarding skill I’ve ever learned. And there’s nothing wrong with learning to cook – I’m a 22 year old rugby-playing New Zealand university student, and I consider myself to be every bit as rugged and articulate as the next Tom, Dick and Harry.

While I was lucky to have a seemingly mild case of PKU and to not have done too much damage during my more care-free years, I can assure you that life with PKU is far more enlightening and interesting – and ultimately more rewarding – if you have tale the time to understand the science behind your condition, how it works, what you can eat and how it can affect how you live your life.

Best Wishes

Dan

James doesn’t have food allergies, though. He suffers from PKU, which drastically limits his diet for the rest of his life. PKU means James’ body can’t metabolize phenylalanine, one of the building blocks of protein.

A highly restricted diet keeps him healthy and experts hope dramatic improvements in treatments could become a reality in his lifetime.

James loves football and bowling. He has the trophies and medals to prove it. In practically all respects, he’s a healthy, happy little boy. His strength is an inspiration to me and I’m his mum!!

His favorite food is burgers and chips and nuggets, special meatless versions of course. In all his six years, James has never eaten meat. And every bite of food he eats is extremely low in protein. Every bite is carefully weighed and measured.

I am very stringent, I know I am but you don’t cheat; you really have to stick to the diet every day to maintain his blood levels and healthy functioning all around.

Many of his friends think he has food allergies. Many ask why he needs to weigh everything he eats.

He tells them we weigh them because he’s on a special diet called PKU. ‘What’s PKU?’” is the standard response to this.

Unchecked, PKU could cause severe mental retardation and seizures. A special formula provides all the protein he needs without the one he doesn’t. Of course, this is a bit hard for a six-year-old to explain to other kids!!

There are always events and fundraising functions for people like James, so please get involved if you get the chance and who knows what the future holds!

James isn’t too concerned about the future though. He’s content with the present.

Gathering the group together in one place not only gave us a chance to demonstrate some great ideas on cooking for a low protein diet, but also gave the HCPs a chance to interact with each other, work together and share their thoughts in a relaxed, fun environment.

Mona Taylor, a professional home economist who has a lot of experience in this specialised area led the day along with Julia Chalkley, a professional chef. This wasn’t just a ‘watch and learn’ day. After each recipe demonstration, the attendees were sent away to their own workstations to ‘give it a go’ themselves. As the aim of this day was for the HCPs to take these tips back to their own clinics, it was vital for them to be comfortable with doing these recipes with their patients without the support of our cooking experts.

The day concentrated on simple recipe ideas such as preparing pasta, but also gave great tips and advice on baking breads and tartlets. As baking can be quite a tricky process at the best of times, Mona and Julia showed the group just how easy it is to use Loprofin baking products and the great results they can achieve with them.

The feedback from the day was very positive, not only was fun had by all, but the ideas taken away from the day will be of great benefit to all those people who follow a restricted diet. As anyone following a restricted diet knows, adherence can be a major issue due to a lack of choice on the diet. Our aim is to show people on the diet that these restrictions need not be the obstacle they appear, and thus improve their chances of sticking to it.

There were times when they would use it as a hold over me, particularly when we were fighting. They would chant “we can eat chocolate but Julie can’t…”. I can still picture them doing it. I would get angry, although I don’t think it was because I couldn’t eat chocolate, it was more because they were picking on me. I have to admit I did try chocolate at an early age but I never liked the taste. They were good to me overall and they always looked out for me, some times too much.

Eating out was fun, we would get some funny looks from people as we brought out the scales to weigh the fries and other stuff. Overall this was rare: we did have a game to come up with the best reason for doing it. Once we convinced this old couple that I was a space cadet from NASA getting ready for my first flight!!

As time went on I did start to rebel, this was mainly in my late teen years. I got a real liking for McDonalds! I think I did suffer though… my grades went down and I put on weight.

Now that I am older I am back on my special diet: I would like to start a family in the near future and its real important for the pregnancy phase.

Well that ends my little blog, I hope it was of some interest to you. Just remember, even though you have PKU you can still live your life.

I met Jill on a night out, and it was about a week or two before PKU was a topic of conversation. I remember Jill came round one day for a BBQ and to meet the parents (there wasn’t any Ben Stiller style comic shenanigans…unfortunately). After the initial meet ‘n’ greet, we moved through to the garden where the Lord of the BBQ (my dad) got to work. The chat was moving along until the inevitable question came, “What would you like Jill? Hamburger? Sausage? Chicken? Ribs?” Jill replied and started to explain about phenylketonuria and that she would not be able to have any of the food my dad had lovingly prepared. My mum, ever the parent stepped in and offered a breadroll to make a chip butty only to be told that the bread had protein in too so she’d just stick to a jacket potato and salad. I think my dad thought she was on one of these new age diets and even said “you need some meat on your bones” slightly missing the point (bless!)

I remember thinking, “what can you eat?” and being completely puzzled by the fact that in the 25 years of my life I had never heard of this fenny-kettle-yourear thingy. I spent hours that night looking on the internet but the first problem I discovered was that I did not know how to spell it!! Google is a great tool when you know what you’re looking for, less so when you don’t! Typical! The next time I saw Jill I told her about my attempt to find out more information and my (frankly, schoolboy) spelling, which she found highly entertaining. Jill said it is often just abbreviated to PKU, which I was fairly confident I could spell!!

Back to the internet, PKU spelt correctly. Once I’d realized that it didn’t stand for PeKing University, I found quite a lot of information about this mystery condition (or at least a mystery to me!)The first time I cooked with PKU foods, I thought I would be a mans man and make a manly batch of….fairy cakes, as Jill could not go to the shops and just buy them like I do (not all the time, but they are a nice treat!) Jill’s mum gave me the prescription ingredients, (Low protein flour, egg replacer) and lent me some low protein cookery books. Making the mixture I felt quite confident, thinking “this is not too bad”, “looks like normal cake mixture” and I had a taste out of curiosity! It tasted like normal cake mixture! I put the mixture into little fairy cases and put them into the oven for 15 mins. After 15 mins they looked really pale so not knowing much about what they should look like, I put them back into the oven to brown. I removed them from the oven not long after, and they had turned brown, but were as solid as rocks! I had completely burnt them! I cut the top off of them and inside did not seem too bad as they looked spongy but slightly lighter in colour to what I was use to. I was so determined to make them so I had another go. This time I removed them from the oven when they were still pale but they were firm to touch. This attempt was much better, they tasted not dissimilar to the cakes I normally have. The moment of truth was when Jill tasted one, she was very impressed and ate the lot! Gordon Ramsey watch out! She was only off work sick for a week too (only joking!)

Three years on our relationship was going strong and we decided to purchase a house, so after traipsing through many properties we managed to purchase one. This was a voyage of discovery in many ways as it would be the first time we had lived together and it would be our first attempt of cooking meals together. This was the first time I really worried about Jill and PKU as I had no idea on cooking main meals for myself let alone a PKU diet. PKU cakes and biscuits were fine but a main meal? What would I cook? I did not understand the exchange system or how to calculate protein in food: it was a minefield! Jill was really reassuring and taught me lots though

The best thing though is that Jill is actually quite a talented chef herself, not to my levels you must understand, my beans on toast have a Michelin star, but she’s more than happy to cook up a meal when she gets home as it’s usually a good two hours before me. She’ll do 2 versions, one for herself and protein-i-fied one for me. Usually this means doing a veggie sauce and frying a bit of chicken for me to add to it. I ain’t complaining. Beggars can’t be choosers

I expect my experience will be familiar to some of you and for people that have just become familiar with PKU it will become a second nature to check ingredients. Some of my ex-girlfriends used to count the calories on packaging this one just counts the protein!